变应性肉芽肿性血管炎16例临床表现和长期随访

Churg-Strauss syndrome： clinical features and long-term follow-up of 16 patients

目的提高对变应性肉芽肿性血管炎（CSS）的认识。方法回顾性分析2001至2007年在北京协和医院确诊并住院治疗的16例CSS患者的临床资料。CSS的诊断基于美国风湿病学会的分类标准。结果所有患者均有哮喘。肺脏、外周神经系统和皮肤是最常见的受累脏器。在疾病的活动期，多数患者外周血嗜酸粒细胞和血清总IgE升高。16例患者中，2例在甲泼尼龙冲击治疗后续用泼尼松，逐渐递减，并加环磷酰胺（CTX）；9例泼尼松递减治疗并加CTX；5例单用泼尼松治疗；部分患者还加用了甲氨蝶呤、羟氯喹、硫唑嘌呤、环孢素、雷公藤、静脉注射用免疫球蛋白等辅助治疗。16例患者病情均缓解。出院后2例患者失访，14例患者随访14～76个月，平均42个月。7例维持缓解，其中3例已停药，3例以小剂量激素及免疫抑制剂维持，1例单用小剂量激素维持；6例复发，平均复发1．7次，复发原因均为激素减量或停药，予激素加量并合用CTX后均缓解，其中4例一直以小或中等量激素维持，2例以中等量激素及CTX维持；1例1年后因家庭纠纷自杀。结论CSS的临床表现和病程个体差异很大，症状可较轻，也可危及生命；糖皮质激素及CTX治疗有效；部分病例虽有复发，但总体预后较好。

Objective To enhance the knowledge about Churg-Stranss syndrome （CSS）. Methods The clinical data of 16 CSS patients were analyzed. Follow-up was conducted for 42 （ 14 - 76） months. Results All patients had the symptom of asthma. As in other case series, the lung, peripheral nervous system, and skin were the most commonly involved organs. During the active stage of the disease, most of the patients exhibited peripheral blood eosinophilia and elevated serum total IgE level. Two patients were treated with pulse treatment of methylprednisolone followed by prednisone with tapering dose and cyclophosphamide, nine patients were treated with corticosteroids and cyclophosphamide, and the others were treated with corticosteroids alone. Several patients were treated with the following drugs in combination： methotrexate, hydroxychloroquine, azathioprim, ciclosporin, common Threewingnut root, and intravenous injection of IgG. Symptom relief was seen in all patients after treatment. Two patients were lost to follow-up. Seven patients showed sustained remission, of which 3 had discontinued the medication, 3 were treated with low-dose corticosteroids and immunity inhibitors, and 1 continued to be treated with low-dose corticosteroids. Six patients suffered from relapse 1.7 times due to withdrawal of corticosteroid treatment or tapering of corticosteroid dose and they regained remission once more after increasing the corticosteroid dose and combination of cyclophosphamide. Four of these 6 patients were treated with corticosteroids of low or medium dose as maintenance doses, and the other 2 with medium-dose corticosteroids and cyclophosphamide. One patient committed suicide because of family dissension 1 year after treatment. Conclusions The clinical manifestations and course of CSS vary considerably. Its clinical manifestations may range from mild symptoms to life-threatening conditions. Corticosteroids and cyclophosphamide are both effective remedies. Despite relapses in several cases, the general prognosis is good.